Acquired hemophilia in a 7‐year‐old girl successfully treated with recombinant FVIIA and steroids: A case report

Giuffrida, Gaetano; Markovic, Uroš; Parisi, Maria Giovanna; Nicolosi, Daniela; Calafiore, Valeria

doi:10.1002/ccr3.3588

Cited by 1 publication

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“…Oboljeli od prirođenih koagulopatija rijetko krvare iz sluznice mokraćnog sustava, dok je makrohematurija izuzetno rijetka kod stečenih koagulopatija (5,6). (12,13). Za razliku od prirođene hemofilije A, stečena se prezentira krvarenjima u kožu, mišiće, gastrointestinalni i mokraćni sustav te životno ugrožavajućima krvarenjem u središnji živčani sustav i u retroperitoneum, dok krvarenja u zglobove nisu tipična za ovaj poremećaj (12,13,14).…”

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Stečena koagulopatija u trogodišnjegdječaka s makrohematurijom– prikaz bolesnika

Malić Tudor,

Armanda

2024

Paediatr Croat

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Acquired coagulopathy is a rare, potentially life-threatening disorder which should be suspected if acute bleeding in patients with a negative previous hemorrhagic diathesis is encountered. This disease arises due to the formation of antibodies against clotting factors in a previously healthy child or adult, which specifically partially or completely neutralize the procoagulant activity of clotting factors or accelerate their removal from the circulating blood, reducing their plasma concentrations and increasing the bleeding tendency. The aforementioned disorder should be confirmed by determining global coagulation times, concentrations of individual clotting factors, and determining the presence of specific or non-specific antibodies. Timely treatment aimed at prompt stopping of bleeding and eradicating the resulting inhibitors will prevent the unfavorable outcome. It is recommended to continue monitoring patients in the outpatient setting with determination of PT and APTT for a year, because in 20% of cases a relapse of the disease occurs, although more often in adults. In this paper, we presented a three-year-old boy in whom we determined prolonged global coagulation times and the presence of non-specific inhibitors of the coagulation factors F II, F IX, F XI and F XII through the diagnostic workup of macrohematuria. The treatment with fresh frozen plasma and corticosteroids resulted in clinical improvement and disease remission. Acquired coagulopathy is a very rare disorder in childhood with scarce literature data. The aim of this paper is to demonstrate the possible causes, clinical course and treatment of this life-threatening disorder in children

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“…(12,13). Za razliku od prirođene hemofilije A, stečena se prezentira krvarenjima u kožu, mišiće, gastrointestinalni i mokraćni sustav te životno ugrožavajućima krvarenjem u središnji živčani sustav i u retroperitoneum, dok krvarenja u zglobove nisu tipična za ovaj poremećaj (12,13,14).…”

Section: Raspravaunclassified

Stečena koagulopatija u trogodišnjegdječaka s makrohematurijom– prikaz bolesnika

Malić Tudor,

Armanda

2024

Paediatr Croat

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Acquired hemophilia in a 7‐year‐old girl successfully treated with recombinant FVIIA and steroids: A case report

Abstract: Acquired hemophilia should be evaluated in pediatric patients with bleeding and isolated prolonged aPTT. Immunosuppressive treatment should be initiated even in minor bleedings. Bypassing agents like rFVIIa can be used in children with success.

Cited by 1 publication

References 18 publications

Stečena koagulopatija u trogodišnjegdječaka s makrohematurijom– prikaz bolesnika

Stečena koagulopatija u trogodišnjegdječaka s makrohematurijom– prikaz bolesnika

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